Outline

Objective: While multiple primary intracranial neoplasms of the same histological type are not unusual in neurosurgical practice, those of different histological origin are rarely encountered. Whereas tumours of the pineal region by itself are uncommon, its coexistence with a pituitary adenoma has never been reported in the literature. Therefore, the authors present the first case of a pituitary adenoma associated with a concurrent pleomorphic pineocytoma.

Methods: A 52-year-old male patient presented with a 5-year history of headache. The neurological and endocrinological examination demonstrated signs and symptoms of occlusive hydrocephalus and anterior pituitary insufficiency. A preoperative magnetic resonance imaging revealed two mass lesions of the sellar and pineal region causing obstructive hydrocephalus. Microsurgical removal of the pituitary tumour via a transnasal transphenoidal approach and exposure to the pineal neoplasm by a supracerebellar infratentorial access after endoscopic third ventriculostomy and biopsy with histopathological examination confirmed the diagnosis of a presumptive pituitary adenoma associated with an unsuspected pleomorphic pineocytoma and led to resolution of the patient’s symptoms. Recovery was uneventful, and at 1-year follow-up, there were no signs of tumour progression.

Results: A unique case of two synchronous intracranial tumours of different histology and location is presented. This exceptional combination of pituitary adenoma associated with pineocytoma has not been documented previously. A microsurgical transnasal transspenoidal approach combined with a supracerebellar infratentorial access to the sellar and pineal regions were successfully applied for the treatment of both intracranial space-occupying lesions.

Conclusions: The simultaneous development of histological different primary brain tumours is rare and its preoperative diagnosis could be difficult, especially in tumours occurring concurrently and at different locations. Furthermore, the histological behaviour is still being defined, as is the prognosis of the synchronous different tumour entities. Thus, treatment options in general remain controversial. The current case is of note not only for the high rarity that it represents, but also for its highlighting of the differential diagnostic and therapeutic challenges faced by neurosurgeons.